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Biomedical subjects

T Kudoh

Publications and source records attributed to T Kudoh.

At least 109 records · Page 6Linked to original sources
PubMed1985

Comparison of enzyme linked immunosorbent assay and enzyme linked fluorescence immunoassay for detection of antibodies against Chlamydia trachomatis.

An enzyme linked fluorescence immunoassay (ELFA) has been evaluated for the detection of antibodies against Chlamydia trachomatis. Reticulate bodies and elementary bodies from C trachomatis L2/434 strain were used as antigens. An enzyme linked immunosorbent assay (ELISA) has also been evaluated using the same antigens. Results obtained by ELISA and ELFA for human sera with these two antigens were compared with each other and with the results obtained by a micro-immunofluorescence (micro-IF) test. Serum IgG antibodies against C trachomatis L2 reticulate bodies and elementary bodies were found in 32 (20.0%) and 11 (6.9%), respectively, of 160 serum samples from pregnant women by the micro-IF test (titre greater than or equal to 1/32). All of these 32 pregnant women had IgG antibodies to C trachomatis reticulate bodies (titre greater than or equal to 1/100), whereas 20 (12.5%) had IgG antibodies to elementary bodies in the ELISA. On the other hand, 25 (15.6%) and 19 (11.9%) of them had IgG antibodies to C trachomatis L2 reticulate bodies and elementary bodies, respectively, by the ELFA (titre greater than or equal to 1/500).

Antibodies, Bacterial↗
PubMed1984

Antithromboembolic treatment after cardiac valve replacement.

Antithrombotic medication is effective in the prevention of thromboembolic complications after valve replacement. Therapy with oral anticoagulant (warfarin), platelet inhibitor drugs and long-term warfarin administration an have good clinical results. However, thrombosis may occur in the early postoperative period, when the oral administration of warfarin is impossible. We have evaluated treatment with urokinase, low-dose heparin and dipyridamole, administered intravenously, instead of warfarin, during the early postoperative period. This procedure was carried out in 30 patients, among whom there was no evidence of thrombosis or of such side-effects as bleeding or a marked tendency to bleed.

Aortic Valve↗
PubMed1983

Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases.

The metabolism of [stearoyl-1-14C]- and [choline-methyl-14C]sphingomyelin, [stearoyl-1-14C]ceramide-1-phospho-N,N-dimethylethanolamine (demethylsphingomyelin) and [choline-methyl-14C]phosphatidylcholine was measured 1, 3 and 5 days after uptake from the media of cultured skin fibroblasts. This was done to measure the relative contributions of lysosomal sphingomyelinase and plasma membrane phosphocholine transferase on the metabolism of sphingomyelin, a component of all cell membranes. By using cell lines from controls and from patients with Niemann-Pick disease and other lysosomal storage diseases, it was concluded that a significant portion (10-15%) of the observed degradation of sphingomyelin is due to exchange of the phosphocholine moiety producing phosphatidylcholine. Although cell lines from type A and B Niemann-Pick disease have only 0-2% of lysosomal sphingomyelinase activity measured in vitro, three cell lines from type B Niemann-Pick disease could metabolize 54.4% of the labeled sphingomyelin by day 3 while cell lines from type A Niemann-Pick disease could only metabolize 18.5% by day 3. This compares to 86.7% metabolized in control cells by day 3. Cells from one patient with juvenile Niemann-Pick disease and one with type D Niemann-Pick disease metabolized sphingomyelin normally while cells from two other patients with juvenile or type C Niemann-Pick disease could only metabolize 58.2% by day 3. Cells from patients with I-cell disease and 'lactosylceramidosis' also demonstrated decreased metabolism of sphingomyelin (55.1 and 54.9% by day 3, respectively). Cells from the patient with Farber disease accumulated [14C]stearic acid-labeled ceramide produced from [14C]sphingomyelin. Studies with choline-labeled sphingomyelin and phosphatidylcholine demonstrated that phosphocholine exchange takes place in either direction in the cells, and this is normal in Niemann-Pick disease. Studies in cells from patients with all clinical types of sphingomyelinase deficiency have led to new methods for diagnosis and prognosis and to a better understanding of sphingomyelin metabolism.

Cell Line↗
PubMed1983

Biochemical studies in a patient with subacute neuropathic Gaucher disease without visceral glucosylceramide storage.

Autopsy samples were obtained from a 12.5-year-old girl who died with a neurologic disorder consisting of myoclonus, myoclonic epilepsy, spasticity, strabismus, and mild mental retardation but no hepatosplenomegaly. Studies in leukocytes, cultured skin fibroblasts, brain, liver, and spleen of this patient revealed glucosylceramide beta-glucosidase (EC 3.2.1.45, glucocerebrosidase) activity about 10% of controls, and well in the range found in samples from Gaucher disease patients. Extraction of the lipids from liver and spleen with chloroform-methanol (2:1) did not show accumulation of glucosylceramide or other lipid. Examination of the lipids in brain by high performance liquid chromatography revealed the presence of glucosylceramide, which is not found in brain samples from controls. Pathologic examination of the liver and spleen revealed no evidence of Gaucher disease. The brain showed many degenerative lesions and loss of neurons. There was no complementation of glucocerebrosidase activity when the cells from this patient were hybridized with cells from patients with Type 1 or Type 2 Gaucher disease. The reason for the lack of glucosylceramide storage in the liver and spleen has not been determined.

Autopsy↗
PubMed1982

A case of type 2 Gm1-gangliosidosis with long survival.

An 11-year-old boy with type 2 GM1-gangliosidosis was presented, providing further evidence for the clinical and biochemical heterogeneity of the disease. The patient had several characteristics of type 2 GM1-gangliosidosis, but was different from so-called typical type 2 GM1-gangliosidosis from the point of view of survival and the degree of GM1-ganglioside accumulation. GM1-gangliosidosis was diagnosed by absence of beta-galactosidase activity in leukocytes and the parents had the enzyme levels of heterozygotes. However, the amount of the brain GM1-ganglioside was accumulated to a less degree in comparison with that of typical type 2 GM1-gangliosidosis, though the activity of GM1-beta-galactosidase in the brain was deficient to the same degree as in the typical case.

Child↗
PubMed1982

Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts.

[(14)C]Stearic acid-labeled cerebroside sulfate (CS) was presented to cultured skin fibroblasts in the media. After endocytosis into control cells 86% was readily metabolized to galactosylceramide, ceramide, and stearic acid, which was reutilized in the synthesis of the major lipids found in cultured fibroblasts. Uptake and metabolism of the [(14)C]CS into cells from typical and atypical patients and carriers of metachromatic leukodystrophy (MLD), Krabbe disease, and Farber disease were observed. Cells from patients with late infantile MLD could not metabolize the CS at all, while cells from an adult MLD patient and from a variant MLD patient could metabolize approximately 40 and 15%, respectively, of the CS taken up. These results are in contrast to the in vitro results that demonstrated a severe deficiency of arylsulfatase A in the late infantile and adult patient and a partial deficiency (21-27% of controls) in the variant MLD patient. Patients with Krabbe disease could metabolize nearly 40% of the galactosylceramide produced in the lysosomes from the CS. This is in contrast to the near zero activity for galactosylceramidase measured in vitro. Carriers of Krabbe disease with galactosylceramidase activity near half normal in vitro and those with under 10% of normal activity were found to metabolize galactosylceramide in cells significantly slower than controls. This provides a method for differentiating affected patients from carriers with low enzyme activity in vitro. Cells from patients with Farber disease could catabolize only approximately 15% of the ceramide produced from galactosylceramide. This technique provides a method for the identification of typical and atypical patients and carriers of three genetic diseases using one substrate.

Amidohydrolases↗
PubMed1981

Vitamin D dependent rickets: decreased sensitivity to 1,25-dihydroxyvitamin D.

A patient with vitamin D dependent rickets with decreased sensitivity to 1,25-Dihydroxyvitamin D was observed. She suffered from bone pain of two years duration beginning at 12 years of age and was found to be suffering from hypocalcemia, secondary hyperparathyroidism and osteomalacia. Laboratory findings revealed normal serum 25-hydroxyvitamin D (27 ng/ml) and markedly elevated serum 1,25-dihydroxy-vitamin D (131.9 pg/ml). The hypocalcemia was refractory in spite of administration of 25,000 units of vitamin D2, but therapy with high doses of oral la-hydroxyvitamin D3 resulted in significant elevation of the serum calcium level. The clinical findings and course of the patient's disease were quite different from those of other patients with vitamin D dependent rickets reported by other authors.

Adolescent↗
PubMed1981

Metabolism of fatty acid-labeled cerebroside sulfate in cultured cells from controls and metachromatic leukodystrophy patients. Use in the prenatal identification of a false positive fetus.

Metachromatic leukodystrophy is the name given to a group of diseases in patients having a deficiency of CS sulfatase activity. The diagnosis usually can be made by using leukocytes, urine, and cultured skin fibroblasts. The low level of enzyme activity can be measured with an artificial substrate, NCS, or suitably labeled CS. In a number of families, healthy carriers of this autosomal recessive disease have been found to have enzyme levels near those of affected patients. We prepared (14)C-stearic acid-labeled CS and studied its metabolism in cultured human cells from patients and controls, In vitro, CS sulfatase requires bile salts to stimulate the enzymatic reaction. The (14)C-CS also can be added to the medium on cultured cells, and its metabolism in the cells can be followed without the addition of bile salts. A child with late infantile MLD was identified by studies on urine and leukocytes. Studies on leukocytes from the parents revealed a very low enzyme level in the father (false positive) and a typical carrier level in the mother. A pregnancy in this family was monitored, and in vitro studies on cultured AFC revealed low CS and NCS sulfatase levels. However, the addition of (14)C-CS to the culture medium revealed normal metabolism in these cells. An unaffected fetus was predicted on the basis of the cell feeding studies. The couple elected to abort this pregnancy, and studies on the fetus confirmed it would not have been affected with MLD.

Amniotic Fluid↗
PubMed1981

Niemann-Pick disease type B: prenatal diagnosis and enzymatic and chemical studies on fetal brain and liver.

Patients with Niemann-Pick disease type A have a severe neurovisceral disease caused by a deficiency of lysosomal sphingomyelinase activity in all tissues examined. The patients with the type B form have signs and symptoms related to storage of sphingomyelin in the spleen, liver, and lungs, while neurologically they remain normal. They also have a severe deficiency of lysosomal sphingomyelinase activity in all tissues previously examined. Here the brain and liver of a fetus with Niemann-Pick disease type B are examined for enzymatic anc chemical changes. Despite careful analysis, no measurable lysosomal sphingomyelinase could be measured in either organ. Lipid changes were comparable to those observed in fetuses aborted with Niemann-Pick disease type A. The affected child in this family is now age 3 and remains neurologically normal but continues to show organ enlargement and lung infiltration of lipids. It appears that the lack of neurological involvement in type B patients cannot be due to an obvious presence of significant lysosomal sphingomyelinase activity in brain.

Adult↗
PubMed1980

Niemann-Pick disease: a genetic model in Siamese cats.

Three Siamese cats were found to have a progressive neurological disease that became obvious when they were 4 to 5 months of age. Their brains contained an excess of GM2 and GM3 gangliosides, and their livers a nine- to tenfold excess of sphingomyelin and cholesterol. A total deficiency of lysosomal (pH 5.0) sphingomyelinase was found in the leukocytes, liver, and brain of the cats, although the activity of the microsomal (pH 7.4, magnesium-dependent) sphingomyelinase was normal in brain. These cats appear to have a genetic disease identical to Niemann-Pick disease type A.

Animals↗
PubMed1980

Fluorometric measurement of alpha-L-iduronidase activity using 4-methylumbelliferyl-alpha-L-iduronide.

Using 4-methylumbelliferyl-alpha-L-iduronide as a substrate, alpha-L-iduronidase activity was measured in leukocytes and in lymphoblastoid cells obtained from patients with alpha-L-iduronidase deficiency and from obligate heterozygotes for this disease. There was complete discrimination between alpha-L-iduronide in leukocytes and in lymphoblastoid cells from the patients and controls. However, overlap was observed between values of the activity in the obligate heterozygotes and those in the controls. 4-Methylumbelliferyl-alpha-L-iduronidase activity because of greater sensitivity, easier assay procedure and shorter incubation period.

Female↗
PubMed1980

Activities of N-acetylgalactosamine-6-sulfate sulfatase in liver from two sisters with morquio syndrome.

A 6-sulfated tetrasaccharide obtained by digesting chondroitin-6-sulfate with testicular hyaluronidase was used as a substrate for the determination of N-acetylgalactosamine-6-sulfate sulfatase activity. The activity was not detected in liver obtained from the elder sister with clinically classic Morquio syndrome and 4.7% of the control in liver from the younger sister with the same disease.

Adolescent↗
PubMed1980

An altered hexosaminidase A in the liver affected by Hurler and Hunter syndromes.

Total hexosaminidase (Hex) in the liver obtained from Hurler and Hunter syndromes displayed about ten times higher activity than that in control liver and moreover, its thermostability was significantly increased. On DEAE cellulose column chromatography, Hex A in the liver of those patients was eluted at much higher salt concentration than that required for elution of Hex A from control liver. There was no difference in elution patterns of Sephadex G-200 and Concanavalin A-Sepharose column chromatographies between the altered Hex A and control Hex A. It appears that the glycosaminoglycans accumulating in the liver of those patients are partly bound to Hex A, and that this binding may cause some changes of its properties such as ionic charge and thermostability.

Adolescent↗
PubMed1979

Neuraminidase activity in liver and brain from patients with I-cell disease.

The activity of neuraminidase in liver and brain from I-cell disease (Mucolipidosis II) was investigated. Neuraminidase activities using two substrates [alpha-L-N-acetylneuraminosyl(2 leads to 3)lactose and alpha-L-N-acetylneuraminosyl(2 leads to 6)lactose] were reduced in the supernatant and sedimentable fractions obtained in isotonic KCl. The activity of beta-D-galactosidase was also reduced in the liver; on the other hand, both neuraminidase fractions were normal, although beta-galactosidase activities were markedly reduced. In view of these results, it is suggested that the defect of neuraminidase is not directly responsible for the primary etiology of I-cell disease.

Brain↗